Surgical
Management
of Duplex Ureteroceles
By G. Monfort, J.M. Guys, M. Coquet, K. Roth, C. Louis, and A. Bocciardi
Marseille, France l We reviewed the cases of 95 children with duplex ureteroceles treated in this department over an Il-year period. There were 101 ureteroceles (6 bilateral). Diagnosis and treatment were analyzed. Special attention was paid to newborns screened in utero. We always strove to preserve functional renal tissue whenever possible. In keeping with this goal, three surgical techniques were used: (1) upper pole heminephrectomy; (2) ureterocele excision, bladder neck reconstruction, and ureter reimplantation with or without cutaneous ureterostomy of the upper pole ureter; and (3) endoscopic ureterocele incision. Follow-up studies using x-ray and radionuclide imaging demonstrated satisfactory renal function in 86.6% of patients. These findings support a conservative approach to ureteroceles using endoscopic ureterocele incision as the primary treatment. Lower urinary tract reconstruction may be associated in cases involving urinary tract infection, obstruction or incontinence. Upper pole heminephrectomy should be performed only after functional evaluation following ureterocele incision or cutaneous ureterostomy. Copyright o 1992 by W. 6. Saunders Company INDEX WORDS:
Duplex ureterocele;
heminephrectomy.
U
PPER POLE heminephrectomy is advocated by many surgeons as the primary treatment of duplex ureterocele. l-6 Our experience at the Timone Children’s Hospital suggests that more conservative management allows preservation of functional renal tissue. MATERIALS AND METHODS
Patient Population The records of 95 patients with duplex ureteroceles were reviewed. Bilateral involvement was seen in 6 cases, for a total of 101 ureteroceles. Age ranged from 1 week to 14 years. The male to female ratio was 1 to 3.4. Urinary tract infection was the most common presenting symptom (78% of all patients). Other symptoms included a flank mass (12.5%), abdominal pain (9%), prolapse of the ureterocele (5.7%), incontinence (12.3%). and hematuria (2.3%). In the last 7 years ultrasound has allowed screening of nearly 28% of the patients in utero. Vesicoureteral reflux was noted in 40 patients and involved the contralateral kidney in 7. The patients were divided into two groups. Group A included 35 patients (38 ureteroceles) in whom the upper pole was visualized at
From the Centre Hospitalier Universitaire de La Timone Hopital des Enfants, Marseille, France. Date accepted: February 22, 1991. Address reprint requests to Gerard J. Monfort, MD. Centre Hospitaker Universitaire de La Timone Hopital des Enfants, I, Blvd Jean-MO&in, 13385 Marseille Cedex 5, France. Copyright o 1992 by W.B. Saunders Company 0022.3468192/2705-0025$03.OOlO
634
the first examination by intravenous urogram or DTPA scan. Of these, 6 appeared normal and 32 dilated. Distension of the lower pole collecting system was observed in 13 cases and scarring in 2. Dilatation of the contralateral collecting system was observed in 7 cases. Group B included 60 patients (63 ureteroceles) in whom the upper pole appeared nonfunctional. In 30 ureteroceles ipsilateral lower pole function was normal, in 19 dilatation was observed, in 8 scarring was noted, and in 6 cases the kidney could not be visualized.
Methods In group A preservation of the upper pole was attempted in all patients. In 29 cases (83%). complete lower urinary tract reconstruction was performed. In 11 of these cases, temporary cutaneous ureterostomy was carried out at the same time. If kidney function was judged to be satisfactory, the ureter was reconstructed either by pyelostomy or by pyeloureterostomy (5 cases). If kidney function was unsatisfactory, heminephrectomy was done. In group A, 9 ureteroceles were diagnosed prenatally and underwent endoscopic incision with good results in 7 (78%). In group B conservative management of the lower pole was indicated in 46 cases presenting normal or mildly dilated collecting systems. In 18 cases treated prior to 1980, temporary cutaneous ureterostomy of the upper pole was performed but preservation was possible in only 3 cases (16.6%). If both the upper and lower poles were nonfunctioning, the entire kidney was removed (17 cases, 27%). Since 1980 endoscopic incision has greatly simplified the treatment of duplex ureteroceles. In this series this technique was used as the primary treatment in 14 cases. The decision to remove or conserve the upper pole was taken after 3 months of free drainage. Reflux in the upper pole recurred after endoscopic incision in 5 cases. In 5 newborn or young infants, this technique allowed demonstration of a functioning upper pole (by intravenous pyelography [IVP] visualization and DTPA scan excretions). Of these 5 patients 3 have not required further treatment and 2 have undergone lower tract reconstruction.
RESULTS
In group A, 30 of 38 upper poles were preserved. In group B, partial upper pole heminephrectomy or total nephrectomy was performed in 38 and 17 cases, respectively. In the total series of 101 duplex ureteroceles, total nephrectomy or upper pole heminephrectomy was required 17 and 46 times, respectively, whereas both upper and lower poles were preserved 38 times. Histological examination of 62 resected upper poles showed dysplasia in 43%. In the remaining 57%, the parenchyma had been destroyed by obstruction and chronic infection but no dysplasia was noted. Overall heminephrectomy was used as the sole primary treatment in only 9 cases. In 5 of these cases reconstruction of the lower urinary tract was also required due to obstruction, reflux in the ipsilateral
JournalofPediatric Surgery, Vol27, No 5 (May), 1992: pp 634-638
SURGICAL MANAGEMENT
635
OF DUPLEX URETEROCELES
Table 1. Fair Results After Ureterocele
Treatment
4 Persistent urinary tract infections 1 After isolated heminephrectomy 1 With transient hypertension 2 Urinary tract infections 4 Stenoses of the reimplanted ureter 1 Aeoperation on ureter 1 Nephrostomy 1 Endoscopic incision 1 Nephrectomy (scan: 10%) 3 Partial incontinence 2 With no improvement (too young for surgery) 1 Successful reoperation (vesicoplasty) 1 persistent reflux Nephrectomy (scan: 18%) 1 vesicovaginal fistula after operative closure
lower pole or contralateral renal unit, prolapse, or urinary tract infection. Of the remaining 4 cases in which lower tract reconstruction was not performed, one girl suffered pain and had a documented urinary tract infection 4 years after heminephrectomy. Long-term results in terms of clinical symptoms, radiologic findings, and renal function were good in 82 of the 95 patients in this series. In the remaining 13, results were fair (Table 1). DISCUSSION
Many urologic surgeons propose upper pole heminephrectomy and partial upper ureterectomy as the primary treatment of choice for duplex ureterocele.1-6
However, recurrent symptoms such as vesicoureteral reflux, persistent urinary tract infection, diverticule formation, incontinence, retention, and voiding problems are frequent. The secondary operative rate ranges from 30% to 50% of cases. We, as well as other authors,4,7-g advocate a conservative approach aimed at preserving functioning renal whenever possible. This attitude seems all the more reasonable today when 50% of the cases of duplex ureteroceles are diagnosed in utero before the aggravating effects of urinary infection. In the present series, this conservative approach was successful in 38% of cases in which a functioning upper poles was demonstrated by IVP and radionuclide imaging. In 1978, Hendren and Mitchell wrote “we are inclined to proceed with the lower repair later because the defunctioned ureterocele can cause continued trouble, infection, reflux in adjacent segments, diverticulum formation and partial outlet obstruction.“‘O The case presented in Fig 1 illustrates these problems. In our experience, collapse of the ureterocele after heminephrectomy did not lead to resolution of reflux. This observation, which has been previously reported, l1 does not validate speculation by Mandell et ali” and Cendron et al.‘” One explanation would be persistent pathology involving the lower pole collecting system, eg, obstructive megaureter. We advocate that endoscopic incision of the ureterocele as a conservative approach to the treatment
Fig 1. Results of upper pole nephrectomy and lower tract reconstruction. in the bottom of the bladder; (C) results after bladder repair (cystography).
(A) Preoperative IVP; (6) preoperative
cystogram: reflux and defect
636
Fig 2.
MONFORT ET AL
Results of conservative treatment
birth; (C) postoperative
(complete reconstruction with temporary cutaneous ureterostomy).
cystogram; (D) postoperative
(A) IVP at birth; (B) cystogram at
IVP.
of duplex ureteroceles. Many investigators have objected to such this approach on the grounds that the associated upper pole is usually dysplasic.3-7~9J1J2In our experience histology showed that destruction of more of than half of the excised upper poles resulted from obstructive changes and infection. No dysplasia was found in these units. This incidence of dysplasia is lower than those reported by Mandell et all2 and
Hennebert et a1.14The issue of long-term malignant transformation of dysplastic elements is controversial. Neither urography nor renal scintigraphy allows reliable assessment of renal function prior to treatment.5 This is particularly true in the newborn. The only sure method is to remove the obstruction before evaluating the kidney or upper pole. Figures 2 and 3
SURGICAL MANAGEMENT
OF DUPLEX URETEROCELES
Fig 3. Results of bladder reconstruction with cutaneous ureterostomy; 35% postoperatively; (D) IVP 1 year postoperatively.
637
later pyelostomy. (A and B) IVP at age 9 months; (C) DTPA left kidney:
638
MONFORT ET AL
present IVP and isotopic scan evidence of functional recovery by upper poles after removal of the obstruction. Endoscopic incision of ureteroceles has been described by us and others. &13,15 This relatively straightforward technique allows early management of septic babies. A careful incision at the base of the ureterocele does not necessarily result in the reflux.1°J6 In the present series reflux developed in only 5 of 14 cases (Fig 4). If reflux does occur, we immediately resect the ureterocele and reimplant the duplex system. The same policy is used in case of huge diverticula or obstruction. Ultrasound allows detection of ureteroceles in utero. This increases the chances of successful conser vative treatment because these patients can be treated before the effects of urinary infection or obstruction Thus, heminephrectomy is not the primary treatmen of choice in such cases. REFERENCES 1. Valayer J, Melin Y: Traitement simplifie de l’urettrocele chez I’enfant. Ann Chir Infant 13:347-354, 1972 2. Belman AB, Filmer RB, King LR: Surgical management of duplication of the collecting system. J Urol 112:316-321, 1974 3. Barrett DM, Malek RS, Kelahs PP: Problems and solutions in surgical treatment of 100 consecutive ureteral duplications in children. J Urol 114:126-130, 1975 4. Sidi AA, Jones P, Hertz M, et al: Experience with surgical management of ectopic ureterocele. Israel J Med Sci 19:530-533, 1983 5. Caldamone AA, Snyder H, Duckett JW: Ureteroceles in children: Follow up of management with upper tract approach. J Urol131:1130-1132, 1984 6. Hanson E, Enger EA, Hjalmas K: Heminephrectomyureterectomy as the sole procedure in ectopic ureterocele in children. Z Kinderchir 39:355-357,1984 7. Heller K, Leng M: Zur operativen therapie ektoper ureterozelen im kindersalter. Pediatr Grenzgeb 22:347-354, 1983 8. Monfort G, Morisson-Lacombe G, Coquet M: Endoscopic treatment of ureteroceles revisited. J Urol133:1031-1033, 1985 9. Heloury Y, Sapin E, Levard G, et al: Traitement des ureteroceles sur duplication pyelo-urcterale. Dtveloppement extravesical chez I’enfant. Ann Ural 19:381-386,1985 10. Hendren WH, Mitchell ME: Surgical correction of ureteroceles. J Urol121:590-597, 1979 11. Cukier J, Petit J, Lanson Y, et al: Traitement simplifie des ureteroceles sur uretbre double. J Ural 125:873-874,198l 12. Mandell J, Colodny AH, Lebowitz R, et al: Ureteroceles in infants and children. J Urol 123:921-926, 1980 13. Cendron J, Melin Y, Valayer J: Traitement simplifie de I’urtterocele avec duplicitt pytlo-ureterale chez I’enfant. Chir Pediatr 21:121-124, 1980 14. Hennebert PN, Wese F, Corm G, et al: L’urettrocele chez l’enfant. 16 cas. Acta Urol Belg 48:370-381, 1980 15. Kroovand RL: Ureterocele. Urol Clin North Am 10:445-449, Fig 4. Results of endoscopic meatotomy (A) IVP at birth, (B) IVP at age 6 months.
of a duplex ureterocele.
1983 16. King LR, Kozlowski JM, Schacht children. JAMA 249:1461-1465, 1983
MJ:
Ureteroceles
in
Management
of Duplex Ureteroceles
By G. Monfort, J.M. Guys, M. Coquet, K. Roth, C. Louis, and A. Bocciardi
Marseille, France l We reviewed the cases of 95 children with duplex ureteroceles treated in this department over an Il-year period. There were 101 ureteroceles (6 bilateral). Diagnosis and treatment were analyzed. Special attention was paid to newborns screened in utero. We always strove to preserve functional renal tissue whenever possible. In keeping with this goal, three surgical techniques were used: (1) upper pole heminephrectomy; (2) ureterocele excision, bladder neck reconstruction, and ureter reimplantation with or without cutaneous ureterostomy of the upper pole ureter; and (3) endoscopic ureterocele incision. Follow-up studies using x-ray and radionuclide imaging demonstrated satisfactory renal function in 86.6% of patients. These findings support a conservative approach to ureteroceles using endoscopic ureterocele incision as the primary treatment. Lower urinary tract reconstruction may be associated in cases involving urinary tract infection, obstruction or incontinence. Upper pole heminephrectomy should be performed only after functional evaluation following ureterocele incision or cutaneous ureterostomy. Copyright o 1992 by W. 6. Saunders Company INDEX WORDS:
Duplex ureterocele;
heminephrectomy.
U
PPER POLE heminephrectomy is advocated by many surgeons as the primary treatment of duplex ureterocele. l-6 Our experience at the Timone Children’s Hospital suggests that more conservative management allows preservation of functional renal tissue. MATERIALS AND METHODS
Patient Population The records of 95 patients with duplex ureteroceles were reviewed. Bilateral involvement was seen in 6 cases, for a total of 101 ureteroceles. Age ranged from 1 week to 14 years. The male to female ratio was 1 to 3.4. Urinary tract infection was the most common presenting symptom (78% of all patients). Other symptoms included a flank mass (12.5%), abdominal pain (9%), prolapse of the ureterocele (5.7%), incontinence (12.3%). and hematuria (2.3%). In the last 7 years ultrasound has allowed screening of nearly 28% of the patients in utero. Vesicoureteral reflux was noted in 40 patients and involved the contralateral kidney in 7. The patients were divided into two groups. Group A included 35 patients (38 ureteroceles) in whom the upper pole was visualized at
From the Centre Hospitalier Universitaire de La Timone Hopital des Enfants, Marseille, France. Date accepted: February 22, 1991. Address reprint requests to Gerard J. Monfort, MD. Centre Hospitaker Universitaire de La Timone Hopital des Enfants, I, Blvd Jean-MO&in, 13385 Marseille Cedex 5, France. Copyright o 1992 by W.B. Saunders Company 0022.3468192/2705-0025$03.OOlO
634
the first examination by intravenous urogram or DTPA scan. Of these, 6 appeared normal and 32 dilated. Distension of the lower pole collecting system was observed in 13 cases and scarring in 2. Dilatation of the contralateral collecting system was observed in 7 cases. Group B included 60 patients (63 ureteroceles) in whom the upper pole appeared nonfunctional. In 30 ureteroceles ipsilateral lower pole function was normal, in 19 dilatation was observed, in 8 scarring was noted, and in 6 cases the kidney could not be visualized.
Methods In group A preservation of the upper pole was attempted in all patients. In 29 cases (83%). complete lower urinary tract reconstruction was performed. In 11 of these cases, temporary cutaneous ureterostomy was carried out at the same time. If kidney function was judged to be satisfactory, the ureter was reconstructed either by pyelostomy or by pyeloureterostomy (5 cases). If kidney function was unsatisfactory, heminephrectomy was done. In group A, 9 ureteroceles were diagnosed prenatally and underwent endoscopic incision with good results in 7 (78%). In group B conservative management of the lower pole was indicated in 46 cases presenting normal or mildly dilated collecting systems. In 18 cases treated prior to 1980, temporary cutaneous ureterostomy of the upper pole was performed but preservation was possible in only 3 cases (16.6%). If both the upper and lower poles were nonfunctioning, the entire kidney was removed (17 cases, 27%). Since 1980 endoscopic incision has greatly simplified the treatment of duplex ureteroceles. In this series this technique was used as the primary treatment in 14 cases. The decision to remove or conserve the upper pole was taken after 3 months of free drainage. Reflux in the upper pole recurred after endoscopic incision in 5 cases. In 5 newborn or young infants, this technique allowed demonstration of a functioning upper pole (by intravenous pyelography [IVP] visualization and DTPA scan excretions). Of these 5 patients 3 have not required further treatment and 2 have undergone lower tract reconstruction.
RESULTS
In group A, 30 of 38 upper poles were preserved. In group B, partial upper pole heminephrectomy or total nephrectomy was performed in 38 and 17 cases, respectively. In the total series of 101 duplex ureteroceles, total nephrectomy or upper pole heminephrectomy was required 17 and 46 times, respectively, whereas both upper and lower poles were preserved 38 times. Histological examination of 62 resected upper poles showed dysplasia in 43%. In the remaining 57%, the parenchyma had been destroyed by obstruction and chronic infection but no dysplasia was noted. Overall heminephrectomy was used as the sole primary treatment in only 9 cases. In 5 of these cases reconstruction of the lower urinary tract was also required due to obstruction, reflux in the ipsilateral
JournalofPediatric Surgery, Vol27, No 5 (May), 1992: pp 634-638
SURGICAL MANAGEMENT
635
OF DUPLEX URETEROCELES
Table 1. Fair Results After Ureterocele
Treatment
4 Persistent urinary tract infections 1 After isolated heminephrectomy 1 With transient hypertension 2 Urinary tract infections 4 Stenoses of the reimplanted ureter 1 Aeoperation on ureter 1 Nephrostomy 1 Endoscopic incision 1 Nephrectomy (scan: 10%) 3 Partial incontinence 2 With no improvement (too young for surgery) 1 Successful reoperation (vesicoplasty) 1 persistent reflux Nephrectomy (scan: 18%) 1 vesicovaginal fistula after operative closure
lower pole or contralateral renal unit, prolapse, or urinary tract infection. Of the remaining 4 cases in which lower tract reconstruction was not performed, one girl suffered pain and had a documented urinary tract infection 4 years after heminephrectomy. Long-term results in terms of clinical symptoms, radiologic findings, and renal function were good in 82 of the 95 patients in this series. In the remaining 13, results were fair (Table 1). DISCUSSION
Many urologic surgeons propose upper pole heminephrectomy and partial upper ureterectomy as the primary treatment of choice for duplex ureterocele.1-6
However, recurrent symptoms such as vesicoureteral reflux, persistent urinary tract infection, diverticule formation, incontinence, retention, and voiding problems are frequent. The secondary operative rate ranges from 30% to 50% of cases. We, as well as other authors,4,7-g advocate a conservative approach aimed at preserving functioning renal whenever possible. This attitude seems all the more reasonable today when 50% of the cases of duplex ureteroceles are diagnosed in utero before the aggravating effects of urinary infection. In the present series, this conservative approach was successful in 38% of cases in which a functioning upper poles was demonstrated by IVP and radionuclide imaging. In 1978, Hendren and Mitchell wrote “we are inclined to proceed with the lower repair later because the defunctioned ureterocele can cause continued trouble, infection, reflux in adjacent segments, diverticulum formation and partial outlet obstruction.“‘O The case presented in Fig 1 illustrates these problems. In our experience, collapse of the ureterocele after heminephrectomy did not lead to resolution of reflux. This observation, which has been previously reported, l1 does not validate speculation by Mandell et ali” and Cendron et al.‘” One explanation would be persistent pathology involving the lower pole collecting system, eg, obstructive megaureter. We advocate that endoscopic incision of the ureterocele as a conservative approach to the treatment
Fig 1. Results of upper pole nephrectomy and lower tract reconstruction. in the bottom of the bladder; (C) results after bladder repair (cystography).
(A) Preoperative IVP; (6) preoperative
cystogram: reflux and defect
636
Fig 2.
MONFORT ET AL
Results of conservative treatment
birth; (C) postoperative
(complete reconstruction with temporary cutaneous ureterostomy).
cystogram; (D) postoperative
(A) IVP at birth; (B) cystogram at
IVP.
of duplex ureteroceles. Many investigators have objected to such this approach on the grounds that the associated upper pole is usually dysplasic.3-7~9J1J2In our experience histology showed that destruction of more of than half of the excised upper poles resulted from obstructive changes and infection. No dysplasia was found in these units. This incidence of dysplasia is lower than those reported by Mandell et all2 and
Hennebert et a1.14The issue of long-term malignant transformation of dysplastic elements is controversial. Neither urography nor renal scintigraphy allows reliable assessment of renal function prior to treatment.5 This is particularly true in the newborn. The only sure method is to remove the obstruction before evaluating the kidney or upper pole. Figures 2 and 3
SURGICAL MANAGEMENT
OF DUPLEX URETEROCELES
Fig 3. Results of bladder reconstruction with cutaneous ureterostomy; 35% postoperatively; (D) IVP 1 year postoperatively.
637
later pyelostomy. (A and B) IVP at age 9 months; (C) DTPA left kidney:
638
MONFORT ET AL
present IVP and isotopic scan evidence of functional recovery by upper poles after removal of the obstruction. Endoscopic incision of ureteroceles has been described by us and others. &13,15 This relatively straightforward technique allows early management of septic babies. A careful incision at the base of the ureterocele does not necessarily result in the reflux.1°J6 In the present series reflux developed in only 5 of 14 cases (Fig 4). If reflux does occur, we immediately resect the ureterocele and reimplant the duplex system. The same policy is used in case of huge diverticula or obstruction. Ultrasound allows detection of ureteroceles in utero. This increases the chances of successful conser vative treatment because these patients can be treated before the effects of urinary infection or obstruction Thus, heminephrectomy is not the primary treatmen of choice in such cases. REFERENCES 1. Valayer J, Melin Y: Traitement simplifie de l’urettrocele chez I’enfant. Ann Chir Infant 13:347-354, 1972 2. Belman AB, Filmer RB, King LR: Surgical management of duplication of the collecting system. J Urol 112:316-321, 1974 3. Barrett DM, Malek RS, Kelahs PP: Problems and solutions in surgical treatment of 100 consecutive ureteral duplications in children. J Urol 114:126-130, 1975 4. Sidi AA, Jones P, Hertz M, et al: Experience with surgical management of ectopic ureterocele. Israel J Med Sci 19:530-533, 1983 5. Caldamone AA, Snyder H, Duckett JW: Ureteroceles in children: Follow up of management with upper tract approach. J Urol131:1130-1132, 1984 6. Hanson E, Enger EA, Hjalmas K: Heminephrectomyureterectomy as the sole procedure in ectopic ureterocele in children. Z Kinderchir 39:355-357,1984 7. Heller K, Leng M: Zur operativen therapie ektoper ureterozelen im kindersalter. Pediatr Grenzgeb 22:347-354, 1983 8. Monfort G, Morisson-Lacombe G, Coquet M: Endoscopic treatment of ureteroceles revisited. J Urol133:1031-1033, 1985 9. Heloury Y, Sapin E, Levard G, et al: Traitement des ureteroceles sur duplication pyelo-urcterale. Dtveloppement extravesical chez I’enfant. Ann Ural 19:381-386,1985 10. Hendren WH, Mitchell ME: Surgical correction of ureteroceles. J Urol121:590-597, 1979 11. Cukier J, Petit J, Lanson Y, et al: Traitement simplifie des ureteroceles sur uretbre double. J Ural 125:873-874,198l 12. Mandell J, Colodny AH, Lebowitz R, et al: Ureteroceles in infants and children. J Urol 123:921-926, 1980 13. Cendron J, Melin Y, Valayer J: Traitement simplifie de I’urtterocele avec duplicitt pytlo-ureterale chez I’enfant. Chir Pediatr 21:121-124, 1980 14. Hennebert PN, Wese F, Corm G, et al: L’urettrocele chez l’enfant. 16 cas. Acta Urol Belg 48:370-381, 1980 15. Kroovand RL: Ureterocele. Urol Clin North Am 10:445-449, Fig 4. Results of endoscopic meatotomy (A) IVP at birth, (B) IVP at age 6 months.
of a duplex ureterocele.
1983 16. King LR, Kozlowski JM, Schacht children. JAMA 249:1461-1465, 1983
MJ:
Ureteroceles
in
